Distinct Laboratory and Clinical Features of Secondary Hemophagocytic Lymphohistiocytosis in Pediatric Visceral Leishmaniasis: A Retrospective Analysis of 127 Children in Andalusia, Spain (2004–2019)

• Published in: The Pediatric infectious disease journal Vol. 40; no. 6; pp. 525 - 530
• Main Authors: López Marcos, María, Ruiz Sáez, Beatriz, Vílchez Pérez, Juan Salvador, Moreno Pérez, David, Carazo Gallego, Begoña, Falcón Neyra, Lola, Goycochea Valdivia, Walter Alfredo, Obando Santaella, Ignacio, Lucena Soto, José Manuel, Díaz Martos, Julio, Santos Pérez, Juan Luis, Rodríguez Benjumea, Margarita, Angulo González de Lara, Raquel, Sánchez Codez, Mª Isabel, Peromingo Matute, Estrella, Cruz Cañete, Marta, Calviño Molinero, Jennifer, Hurtado Mingo, Ángela, Lendínez Molinos, Francisco, Medina Claros, Antonio F., Muñoz Vilches, Mª José, Neth, Olaf, Olbrich, Peter
• Format: Journal Article
• Language: English
• Published: United States Lippincott Williams & Wilkins 01-06-2021
• Subjects: Amphotericin B - therapeutic use; Antiprotozoal Agents - therapeutic use; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Laboratories; Leishmaniasis, Visceral - complications; Leishmaniasis, Visceral - drug therapy; Leishmaniasis, Visceral - epidemiology; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - drug therapy; Lymphohistiocytosis, Hemophagocytic - epidemiology; Lymphohistiocytosis, Hemophagocytic - pathology; Male; Retrospective Studies; Spain - epidemiology; Spain
• ISSN: 0891-3668; 1532-0987
• DOI: 10.1097/INF.0000000000003086

Visceral leishmaniasis (VL) is an endemic in Southern Europe. However, details regarding disease burden, clinical presentations, laboratory markers, management and outcome in children are scarce.
Medical records of children (<14 years) admitted with VL to 10 pediatric units in Andalusia (2004-2019) were retrospectively reviewed. VL diagnosis was based on clinical presentation, serology, microscopy and molecular methods. Diagnosis of secondary hemophagocytic lymphohistiocytosis (sHLH) was established using the hemophagocytic lymphohistiocytosis-2004 criteria.
A total of 127 patients were identified. Median age was 14.5 months; the main clinical presentations were fever and splenomegaly (95.3% each). Cytopenias were the most common laboratory abnormalities. Diagnostics as well as treatment regimens varied over time and the participating centers. Liposomal amphotericin B was prescribed in 97.6%; relapses as well as adverse events were rarely observed (3.1% each). Thirty-seven patients, diagnosed with sHLH required longer hospital admission (P = 0.001), an increased number of platelet (P < 0.006) and red blood cell (P = 0.0001) transfusions and pediatric intensive care unit admission (P = 0.007). Monocytopenia (P = 0.011) and high C-reactive protein levels (P = 0.031), variables not included in the hemophagocytic lymphohistiocytosis-2004 criteria, were associated with sHLH. One patient deceased in the context of the Leishmania infection.
We report data on the largest pediatric VL cohort from Europe, commonly associated with sHLH. Raised C-reactive protein levels and monocytopenia appear to be associated with sHLH. The latter may help to identify these patients and to guide decisions regarding need of additional supportive clinical care and immunomodulatory therapies. The observed high rate of heterogeneity in terms of diagnosis and management warrants the establishment of appropriate guidelines.